BACKGROUND Listeria monocytogenes (L. monocytogenes), a Gram-positive facultatively intracellular bacterium, is the causative agent of human listeriosis. Listeria infection is usually found in immunocompromised patients, including elderly people, pregnant women, and newborns, whereas it is rare in healthy people. L. monocytogenes may cause meningitis, meningoencephalitis, and some very rare and severe complications, such as hydrocephalus and intracranial hemorrhage, which cause high mortality and morbidity worldwide. Up to now, reports on hydrocephalus and intracranial hemorrhage due to L. monocytogenes are few. CASE SUMMARY We herein report a case of rhombencephalitis caused by L. monocytogenes in a 29-year-old man. He was admitted to the hospital with a 2-d history of headache and fever. He consumed unpasteurized cooked beef two days before appearance. His medical history included type 2 diabetes mellitus, and contaminated beef intake 2 d before onset. Cerebrospinal fluid analysis revealed Gram-positive rod infection, and blood culture was positive for L. monocytogenes. Magnetic resonance imaging findings suggested rhombencephalitis and hydrocephalus. Treatment was started empirically and then modified according to the blood culture results. Repeated CT images were suggestive of intracranial hemorrhage. Although the patient underwent aggressive external ventricular drainage, he died of a continuing deterioration of intracranial conditions. CONCLUSION Hydrocephalus, intracranial hemorrhage, and inappropriate antimicrobial treatment are the determinations of unfavorable outcomes.